書誌事項
- タイトル別名
-
- Chronic Respiratory Failure in a Case with Juvenile-Onset Acid .ALPHA.-Glucosidase Deficiency; Successful Therapy with Nasal Intermittent Positive Pressure Ventilation(NIPPV).
この論文をさがす
説明
A 13-year-old boy with juvenile-onset acid a glucosidase deficiency was reported. Proximal muscle weakness including respiratory muscles and scoliosis progressed since nine year of age. He developed nocturnal dyspnea and daytime somnolence at age 13. His arterial blood gas analysis showed hypoxemia (PO, 54.1 mmHg) and hypercapnia (PCO2 72.3mmHg), and spirometry showed significantly decreased vital capacity (%VC 21%). He was treated with nocturnal NIPPV employing a device for delivering bilevel positive airway pressure (Bi-PAP). Nocturnal dyspnea and daytime somnolence rapidly disappeared with nocturnal ventilatory support. Daytime arterial P02 and PCO2 improved after the therapy, namely 74.8 mmHg and 64.1 mmHg respectively. We conclude that NIPPV is a noninvasive and effective therapy for respiratory failure in patients with chronic progressive neuromuscular disorder including acid a glucosidase deficiency.
収録刊行物
-
- 脳と発達
-
脳と発達 29 (1), 51-54, 1997
THE JAPANESE SOCIETY OF CHILD NEUROLOGY
- Tweet
詳細情報 詳細情報について
-
- CRID
- 1390282679530690432
-
- NII論文ID
- 130004183088
- 10005658434
-
- NII書誌ID
- AN0020232X
-
- ISSN
- 18847668
- 00290831
-
- PubMed
- 8986097
-
- 本文言語コード
- ja
-
- 資料種別
- journal article
-
- データソース種別
-
- JaLC
- PubMed
- CiNii Articles
-
- 抄録ライセンスフラグ
- 使用不可
